Emma Joyce

Thank you to Rhonda for sharing her beautiful daughter, Emma Joyce's, story with us!

Emma was born April 20, 2007. Not long after, I had sensed “something” was not right. Emma seemed jumpy, delayed and not able to follow vision wise. At her 2 month immunization appointment, I asked her doctor to take another look at her. She was then referred to the pediatrician. A few weeks later at her appointment, he immediately sent us to Winnipeg Children’s Hospital. She ended up getting admitted for testing, which was to take a couple days. It turned into a very long three weeks. I kept telling the doctors it was like she was having a seizure. Finally they called the neurologist and he didn’t think so, but sent her for an EEG to be sure. Little did I know the next 45 minutes of that day would change our lives forever. They told us we were to wait in the hallway for about 45 minutes. After 15 minutes they called us in to tell us Emma has had 4 seizures since she had been in there. Although I was devastated, I tried to look on the bright side, thinking lots of people have seizures, it can be managed. I did not realize how dead wrong I was. Emma was given a load of Phenobarb to settle the seizures while they could do further testing. She was so sedated, she was out for 3 days, and I was starting to go crazy. She underwent numerous testing, such as EEG (had it on for a few days), MRI, LP, numerous blood tests, PEG Radioactive testing, and that is to name a few. She was having 20 + seizures a day, which were Infantile Spasms, so it just looked like she was jumping or startling while falling asleep, while sleeping or awakening.

She was finally released from the hospital and sent home on ACTH injections, which is a steroid. It worked wonderfully to control seizures, however, you cannot be on it forever. When she stopped the injections, we had numerous trips to ER here in Brandon as she was having seizure clusters ( seizing, brief stop, then seizing again, etc). We have had so many adjustments to her meds.

In June, 2008, her neurologist finally told me what she was diagnosed with, although I am pretty sure he knew before. She has Early Epileptic Encephalopathy of Infancy, otherwise known as Ohtahara Syndrome. It consists of, but by all means not limited to, cortical vision impairment, severe developmental delay (she is like a newborn baby), seizures (very difficult to control with medication) and feeding issues.

In January, 2009, Emma was admitted to WCH to start on the Ketogenic Diet, which it a medical diet often used to control seizures. At that time, she also underwent a feeding study, at my request. Since then she has been restricted to have anything by mouth, as she was putting 80% into her lungs, causing her to have aspirational pneumonia. She was scheduled for G Tube surgery. The night before surgery, she had a fever, which turned out to be the start of another pneumonia. Surgery was canceled and the next date was February 13th. She remained in the hospital all this time. After her surgery we returned home on the 18th of February. It took a lot of getting used to, as I was having to use the feeding pump, IV pole, etc. She adjusted wonderfully, life was great!

March 10th came along and Emma started having severe diarrhea. Her doctor and I figured, probably just a flu bug. Over spring break, we went into Winnipeg as she had appointments with Metabolics, neurology, surgery follow-up, and her pediatrician. They were all clueless as to WHY she would still be having diarrhea. We collected stool samples and sent them away, with no results to show anything. We took her off the Ketogenic Diet; put her on Nutren JR. with pedialyte to see if that worked. It didn’t. At the end of April, she was still the same. We went to the Rehab center for Children clinic to see her feeding specialist. We switched her over to a formula that is the most broken down formula you can get. After 5 days of being on the new formula, Emma was nothing but bones, she dropped weight like crazy. Her eyes were sunken in and she looked absolutely horrible. After having her on straight pedialyte, and no result for the better, I took her back up to the hospital here. The doctor could not figure out why either, especially after getting pedialyte on continual feed. Emma was so dehydrated and malnourished, that her sodium was so high; she was at major risk for a stroke. They admitted her to BGH. After being in hospital 4 days, with still no answers, Emma developed pneumonia again. Her doctor refused to prescribe her an antibiotic, and she was so weak, she kept desating and they finally transferred her to Winnipeg via ambulance. She was admitted to CK3 there, and shortly after, rushed into PICU.

In PICU, seeing Emma for the first time, hooked up to BIPAP, I thought, my God, this is it, this is how I am going to lose her. It is a mother’s worst nightmare. I was standing beside Emma and realized they had her G-Tube hooked up to a drain, to empty out her tummy. I asked the nurse in there, what is that coming out? Yes, it was poop. The next day, she underwent a fluoroscopy, to test where the fluid is going when put into the tube. That is when we finally found out why she had diarrhea in the first place. Her G-tube was in her colon, NOT her tummy! Needless to say, she was very dependent on the BIPAP machine and still very weak. She spent 2 months in PICU and up on the ward. Finally, we were sent home at the end of June, once Emma had recovered wonderfully as she could have. She was sent home on oxygen, and she had an NG and NJ tube for feeding and we were awaiting her surgery. First two surgeries were canceled due to the H1N1 breakout at the hospital in Wpg. All of the PICU beds were full and they would not do Emma’s surgery without a bed In PICU for her after.

Finally, at the end of August she went for surgery. Emma had to get a fundoplication, which is the stomach wrapped around the esophagus to prevent refluxing, repair of the two fistulas (hole in colon and stomach from first tube) and a new G tube. I made the decision for her to have an Epidural instead of Morphine for obvious breathing issues. After her surgery, she was placed back on Bipap, just so they didn’t lose headway with her. The next day, she was wonderful! She came off the BIPAP and was back on her O2 mask. After a few days in PICU, we were sent up to the ward. After a few days and after they were sure everything was fine and her feeds were being tolerated at continual, we came home!! When we came home, it was my job to work Emma up in volume and down to a bolus feed (one feed at a time). She is currently on 4 feeds a day, with each one lasting 2 ½ hours.

She still requires home oxygen, as well as portable when we leave the house. She is always on an oximeter, which shows her heart rate, O2 level, and will alarm when desating. We were so fortunate to get her wheelchair. It allows Emma to be most importantly of all, comfortable. It also allows me to take her out and feed her at the same time, as they put an IV pole on it for her feeding pump and her feeding bag and also allows me to hang her backpack oxygen on it.

We still have numerous appointments to Winnipeg. Emma goes to Neurology, Metabolics, Respiratory, her pediatrician, her ophthalmologist and the Rehabilitation Center for Children (which I also usually have to transport her really large equipment) in Winnipeg throughout the year. She also has a few appointments in Brandon, usually the doctor here, and all of our fun ER trips, which really add up in a year! Her PT/OT/ST come to the house now, as well as her OT from the Canadian National Institute for the Blind.

 

Emma still gets a lot of respiratory infections, due to her swallowing issues, and I’m sure the Trachial Malaysia is part reason. Since being home, Emma has went from a mere 15 pound girl that was first admitted into Brandon last spring, to a very big 41 pound girl!! She is such a strong girl and such a little fighter, and I am so proud and blessed to have her in my life.